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Dr. Pirooz Eghtesadyis a pediatriccardiothoracic surgeonat the Washington University
School of Medicine in St Louis. His research focus is on congenital
heart diseases, with particular emphasis on aortic stenosis and
hypoplastic left heart syndrome. In this episode, Dr.Eghtesadydiscusses the surgical procedure
called a Potts shunt as a treatment option for children who suffer
from pulmonary hypertension.

My name is Pirooz Eghtesady. I'm here at Washington University
in St. Louis or St. Louis Children's Hospital. I've been here for
about seven years. Specifically, I'm a pediatric I'm a
cardiothoracic surgeon.

My connection to the pulmonary hypertension community stems from
the fact that ... I say unfortunately, because that's a reality...
I face a lot of the circumstances when children have no other
choice, no other option and come to lung transplantation. Kind of
correlating to that, was this notion of why aren't we doing the
Potts shunt like they were doing in Europe when I first read about
it. I was fortunate enough to have supportive partners like Dr.
Grady and essentially then it's kind of taken off in that regard.
We've seen, similar to the folks in Europe, some dramatic
response.

The thought process people have had, which I think is actually
on the right track, is that one big distinction in terms of when
the onset of right ventricular failure or right heart failure comes
about. In patients who have the ability to shunt, to be able to
push the blood in the opposite direction, the potential for
maintaining heart function for a long duration of time appears to
be better than patients who don't have that pop-off, if you
will.

When I came here, I started thinking about different ways of
trying to improve the outcomes, both as bridging patients to lung
transplant, as well as just in general improving the outcomes.
First, there was some reticence and some concern about something
that you could say it is experimental not having done it before.
The very first patient was a very, very sick child that I operated
on and was basically on ECMO. It was a patient who had prior
surgeries. It was a very complex operation. Fortunately, we got him
through the surgery, and he did okay for a while, but it wasn't a
longterm solution for him. We then, sort of in that process, we
just kept going and we've now done 14 or 15 patients since I
started doing those about four years, five years, something along
those lines.

It's been known for a long time with any lesion involving the
heart that is some kind of obstruction, so whether it's an
obstruction at the level of the mitral valve or obstruction of the
aortic valve, obstruction of the tricuspid valve, obstruction of
the pulmonary valve, those lesions are not well tolerated, because
the net effect is that they decrease your cardiac output or they
limit your cardiac output.

This is the analogy that I can give you is that the heart is a
system and series. Blood comes into the right side, goes to the
lungs, comes to the left side and gets out of the heart.
Effectively, you've got four valves or four doors along that
pathway. Imagine running through a corridor with four doors. Any
one of those doors doesn't open properly. The net effect of
throughput is limited. If you have a bunch of people trying to get
out of a building that's on fire, wherever the door is not opening
effectively, you're going to get backup of people and that room, if
you will, is going to get really congested. At first, it might be
reasonably tolerated, but the more severe it gets and more people
get crammed in there, before you know it they're banging the wall,
breaking the windows because they need to get out. At some point
the damage that comes becomes irreversible.

That is in contrast to when the door is absent or when there is
leakage, say, any of those valves. That can be very well tolerated.
People can go back and forth. If you have no doors, you can still
get traffic. You can get flow. It's just not as effective. When you
have bad pulmonary hypertension and blood can't get through the
lungs, the consequence is that the right heart starts getting
unhappy and starts pushing in to actually the left side, because
the right heart and left heart hug each other. If you actually look
at it under like sort of a cross section of a heart muscle, the
right heart is kind of wrapped around the left heart. The way those
two interact with each other is like a dance. When they contract
there is, in fact, a specific location where there is three walls,
if you will. We talk about the free wall, the LV free wall, the RV
and then the intraventricular septum which is the wall in between
the right and left. Those interact with each other, and when the
right side gets really pressurized, that septum shifts over and
starts pushing into the left side.

It has two consequences. One, is the fact that the right side is
struggling, but it also starts impacting the left side, so your net
effective cardiac output goes down even more, more so than you
would expect simply because blood is not getting around. The
ventricle, it can build up muscle, but after a time, what happens
when the stress is extreme, the muscle gets replaced with scar
tissue. At that point, when you start getting scar tissue, you
can't really reverse it, whether it's on the right side or it's
left side. Currently, there really isn't a great way.

If people had a massive heart attack, historically, the reason
heart attacks were so deadly was because by the time people would
get blood reperfusing the heart muscle to salvage it, it would take
so long. So much of the muscle would die and get replaced by scar
tissue that you would be crippled from heart failure even though
things had been fixed, if you will, by restoring blood flow. Now,
because treatment is more aggressive and people get to it faster,
in fact, they struggle to have trials to show efficacy of various
treatments because you don't end up in that situation. We have so
much of the heart muscle replaced by scar.

Intervening at any point prior to development of that scar
tissue is really important. Our ability to assess the amount of
scar tissue is very limited. The ways that we can do that are some
of them are indirect using echo and catheterization, but they're
not super precise. With MRI, if it's really bad, you can tell.
Again, you really can't assess when it's in the early phases of it.
It makes it tough.

The notion of being able to protect the heart, protecting the
right ventricle, and the ways that we do it is by looking at echo
and by looking at MRI to make functional measurements. It really is
the only way we can do it, and we try to protect the right side,
the right ventricle. The concept of the Potts shunt is before
you've sort of fallen off that curve, before that process has
happened, you create a pop-off for the right ventricle so it
doesn't have to push as hard at the expense of having some of blood
bypassing the lungs, so it doesn't get oxygen, but it allows the
heart to work more effectively. You improve the overall cardiac
output of the patient, so the ventricular septum no longer shifts
as much so the overall effective heart output, the cardiac output
improves.

Most of the patients who have been here, been here typically for
about two to three weeks, somewhere in that range. Particularly
when you go through a thoracotomy, it's a painful procedure in that
regard and does take some time for rehabilitation. Generally, the
patients have spent about five days in the ICU. It kind of depends
on the condition of the patient, so the patients that are coming in
who are ... and I know this sounds like an oxymoron, but patients
who are in good shape, they do really well. If they're very sick
coming in, then I think that translates into the outcome. We had
one patient who had gone to the catheterization lab and ended up on
ECMO, and they had sent the patient to us on support, and that
patient never left the ICU. It really depends on the situation of
what the patient's condition is coming into the operation. On
average, I would say most patients are in the hospital for about
somewhere around two to three weeks of which maybe a week of that
is in the ICU and the rest of it is kind of rehabilitating, walking
around, things like that.

Please discuss it with your physician, with your pulmonary
hypertension physician. It's my general impression. This is not
specifically for this procedure, but in general, that a lot of
times we wait until the very end. You cannot expect good results
when you have waited too long. My overall impression even with this
relatively limited experience that the younger the patients and the
better condition they are, the better the outcome to the point that
some I've seen them be able to completely come off of
therapies.

It's one of those things that just being aware, gathering as
much information about it. It's a very personal decision. The
family has to sort of weight the risks, benefits. If they're
plugged in with their pulmonary hypertension colleagues, physician
colleagues that are providing guidance and are familiar with the
procedure, that's great. If not, they can always reach out. There's
a number of people now that are developing expertise beyond even
the surgical aspects of this.

Dr. Dunbar Ivy in Colorado, we work with him very closely. We've
had a number of his patients come through. He has a fair amount of
experience about those patients and how they do. I think connecting
with physicians like him, Dr. Grady and various folks, I think it
can be an opportunity to learn more about the procedure. Certainly,
I'd be happy to talk to any family member who is interested or
wants to have more information about the procedure and the details
of it. I think that is what I would want them to know most, that
it's available. The results are promising and sooner than later is
the key. That's what I would say.

About the Podcast

A new podcast series devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. New Episodes every Monday & Thursday.
Through this series of impactful, insightful and, most importantly, hopeful stories from members of the global pulmonary hypertension community, we hope to further the global #phaware conversation as well as to capture, engage and enable misdiagnosed and undiagnosed PH patients because early diagnosis and treatment can mean the difference between life and death.
Topics range from the importance of early diagnosis and global PH awareness, to the impact of clinical trials to how patient support and advocacy are paramount to battling this disease.
Learn more about PH at www.phaware.global